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ISMAEL AL-RAMAHI

TitleAssistant Professor
InstitutionBaylor College of Medicine
DepartmentDepartment of Molecular & Human Genetics
Address1250 MOURSUND STREET
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    Collapse Research 
    Collapse research activities and funding
    R01AG074009     (LICHTARGE, OLIVIER)Sep 15, 2021 - Jun 30, 2026
    NIH
    Decoding the impact of sex differences on Alzheimer's disease risk
    Role: Co-Principal Investigator
    R21NS096395     (AL-RAMAHI, ISMAEL)Sep 1, 2016 - Aug 31, 2019
    NIH
    Behavioral and cell-based complementary screens to identify druggable targets modulating tau protein levels
    Role: Principal Investigator

    Collapse Bibliographic 
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    Publications listed below are automatically derived from MEDLINE/PubMed and other sources, which might result in incorrect or missing publications. Faculty can login to make corrections and additions.
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    PMC Citations indicate the number of times the publication was cited by articles in PubMed Central, and the Altmetric score represents citations in news articles and social media. (Note that publications are often cited in additional ways that are not shown here.) Fields are based on how the National Library of Medicine (NLM) classifies the publication's journal and might not represent the specific topic of the publication. Translation tags are based on the publication type and the MeSH terms NLM assigns to the publication. Some publications (especially newer ones and publications not in PubMed) might not yet be assigned Field or Translation tags.) Click a Field or Translation tag to filter the publications.
    1. Sonn JY, Kim W, Iwanaszko M, Aoi Y, Li Y, Parkitny L, Brissette JL, Weiner L, Al-Ramahi I, Botas J, Shilatifard A, Zoghbi HY. MeCP2 Interacts with the Super Elongation Complex to Regulate Transcription. bioRxiv. 2024 Jul 01. PMID: 39005382; PMCID: PMC11244860.
      Citations:    
    2. Edwards GA, Wood CA, He Y, Nguyen Q, Kim PJ, Gomez-Gutierrez R, Park KW, Xu Y, Zurhellen C, Al-Ramahi I, Jankowsky JL. TMEM106B coding variant is protective and deletion detrimental in a mouse model of tauopathy. Acta Neuropathol. 2024 03 25; 147(1):61. PMID: 38526616.
      Citations: 6     Fields:    Translation:HumansAnimals
    3. Wu T, Deger JM, Ye H, Guo C, Dhindsa J, Pekarek BT, Al-Ouran R, Liu Z, Al-Ramahi I, Botas J, Shulman JM. Tau polarizes an aging transcriptional signature to excitatory neurons and glia. Elife. 2023 May 23; 12. PMID: 37219079; PMCID: PMC10259480.
      Citations: 2     Fields:    Translation:HumansAnimalsCells
    4. Yu M, Ye H, De-Paula RB, Mangleburg CG, Wu T, Lee TV, Li Y, Duong D, Phillips B, Cruchaga C, Allen GI, Seyfried NT, Al-Ramahi I, Botas J, Shulman JM. Functional screening of lysosomal storage disorder genes identifies modifiers of alpha-synuclein neurotoxicity. PLoS Genet. 2023 05; 19(5):e1010760. PMID: 37200393; PMCID: PMC10231792.
      Citations: 1     Fields:    Translation:HumansAnimalsCells
    5. Bourquard T, Lee K, Al-Ramahi I, Pham M, Shapiro D, Lagisetty Y, Soleimani S, Mota S, Wilhelm K, Samieinasab M, Kim YW, Huh E, Asmussen J, Katsonis P, Botas J, Lichtarge O. Functional variants identify sex-specific genes and pathways in Alzheimer's Disease. Nat Commun. 2023 05 13; 14(1):2765. PMID: 37179358; PMCID: PMC10183026.
      Citations: 4     Fields:    Translation:Humans
    6. Edwards GA, Wood CA, Nguyen Q, Kim PJ, Gomez-Gutierrez R, Park KW, Zurhellen C, Al-Ramahi I, Jankowsky JL. TMEM106B coding variant is protective and deletion detrimental in a mouse model of tauopathy. bioRxiv. 2023 Mar 25. PMID: 36993574; PMCID: PMC10055407.
      Citations:    
    7. Han X, Wang W, Ma LH, Al-Ramahi I, Botas J, MacKenzie K, Allen GI, Young DW, Liu Z, Maletic-Savatic M. SPA-STOCSY: An Automated Tool for Identification of Annotated and Non-Annotated Metabolites in High-Throughput NMR Spectra. bioRxiv. 2023 Feb 22. PMID: 36865102; PMCID: PMC9980041.
      Citations:    
    8. Li J, Amoh BK, McCormick E, Tarkunde A, Zhu KF, Perez A, Mair M, Moore J, Shulman JM, Al-Ramahi I, Botas J. Integration of transcriptome-wide association study with neuronal dysfunction assays provides functional genomics evidence for Parkinson's disease genes. Hum Mol Genet. 2023 01 27; 32(4):685-695. PMID: 36173927; PMCID: PMC9896475.
      Citations: 1     Fields:    Translation:AnimalsCells
    9. Benyair R, Giridharan SSP, Rivero-R?os P, Hasegawa J, Bristow E, Eskelinen EL, Shmueli MD, Fishbain-Yoskovitz V, Merbl Y, Sharkey LM, Paulson HL, Hanson PI, Patnaik S, Al-Ramahi I, Botas J, Marugan J, Weisman LS. Upregulation of the ESCRT pathway and multivesicular bodies accelerates degradation of proteins associated with neurodegeneration. Autophagy Rep. 2023; 2(1). PMID: 37064812; PMCID: PMC10101321.
      Citations:    
    10. Kim J, de Haro M, Al-Ramahi I, Garaicoechea LL, Jeong HH, Sonn JY, Tadros B, Liu Z, Botas J, Zoghbi HY. Evolutionarily conserved regulators of tau identify targets for new therapies. Neuron. 2023 03 15; 111(6):824-838.e7. PMID: 36610398.
      Citations: 5     Fields:    Translation:HumansAnimals
    11. Zhang X, Wen X, Al-Ramahi I, Botas J, Lu B, Fu Y. Correction: Inhibition of HIPK3 by AST487 Ameliorates Mutant HTT-Induced Neurotoxicity and Apoptosis via Enhanced Autophagy. Neurosci Bull. 2022 Sep; 38(9):1123. PMID: 35864371; PMCID: PMC9468195.
      Citations:    Fields:    
    12. Lagisetty Y, Bourquard T, Al-Ramahi I, Mangleburg CG, Mota S, Soleimani S, Shulman JM, Botas J, Lee K, Lichtarge O. Identification of risk genes for Alzheimer's disease by gene embedding. Cell Genom. 2022 Sep 14; 2(9). PMID: 36268052; PMCID: PMC9581494.
      Citations: 2     
    13. Lee WS, Al-Ramahi I, Jeong HH, Jang Y, Lin T, Adamski CJ, Lavery LA, Rath S, Richman R, Bondar VV, Alcala E, Revelli JP, Orr HT, Liu Z, Botas J, Zoghbi HY. Cross-species genetic screens identify transglutaminase 5 as a regulator of polyglutamine-expanded ataxin-1. J Clin Invest. 2022 05 02; 132(9). PMID: 35499073; PMCID: PMC9057624.
      Citations:    Fields:    Translation:HumansAnimals
    14. Greco TM, Federspiel JD, Liu JP, Perez AM, Al-Ramahi I, Cantle JP, Carroll JB, Botas J, Zeitlin SO, Cristea IM, Secker C, Ramos ES, Wanker EE. Dynamics of huntingtin protein interactions in the striatum identifies candidate modifiers of Huntington disease. Cell Syst. 2022 04 20; 13(4):304-320.e5. PMID: 35148841; PMCID: PMC9317655.
      Citations:    Fields:    Translation:Animals
    15. Zhang X, Wen X, Al-Ramahi I, Botas J, Lu B, Fu Y. Inhibition of HIPK3 by AST487 Ameliorates Mutant HTT-Induced Neurotoxicity and Apoptosis via Enhanced Autophagy. Neurosci Bull. 2022 Jan; 38(1):99-103. PMID: 34741261; PMCID: PMC8782961.
      Citations:    Fields:    Translation:Humans
    16. Suart CE, Perez AM, Al-Ramahi I, Maiuri T, Botas J, Truant R. Spinocerebellar Ataxia Type 1 protein Ataxin-1 is signaled to DNA damage by ataxia-telangiectasia mutated kinase. Hum Mol Genet. 2021 05 17; 30(8):706-715. PMID: 33772540.
      Citations: 2     Fields:    Translation:HumansAnimalsCells
    17. Onur TS, Laitman A, Zhao H, Keyho R, Kim H, Wang J, Mair M, Wang H, Li L, Perez A, de Haro M, Wan YW, Allen G, Lu B, Al-Ramahi I, Liu Z, Botas J. Downregulation of glial genes involved in synaptic function mitigates Huntington's disease pathogenesis. Elife. 2021 04 19; 10. PMID: 33871358; PMCID: PMC8149125.
      Citations: 2     Fields:    Translation:HumansAnimalsCells
    18. Lee WS, Lavery L, Rousseaux MWC, Rutledge EB, Jang Y, Wan YW, Wu SR, Kim W, Al-Ramahi I, Rath S, Adamski CJ, Bondar VV, Tewari A, Soleimani S, Mota S, Yalamanchili HK, Orr HT, Liu Z, Botas J, Zoghbi HY. Dual targeting of brain region-specific kinases potentiates neurological rescue in Spinocerebellar ataxia type 1. EMBO J. 2021 04 01; 40(7):e106106. PMID: 33709453; PMCID: PMC8013850.
      Citations: 3     Fields:    Translation:HumansAnimalsCells
    19. Kim YW, Al-Ramahi I, Koire A, Wilson SJ, Konecki DM, Mota S, Soleimani S, Botas J, Lichtarge O. Harnessing the paradoxical phenotypes of APOE ?2 and APOE ?4 to identify genetic modifiers in Alzheimer's disease. Alzheimers Dement. 2021 05; 17(5):831-846. PMID: 33576571; PMCID: PMC8247413.
      Citations: 7     Fields:    Translation:HumansAnimals
    20. Huichalaf CH, Al-Ramahi I, Park KW, Grunke SD, Lu N, de Haro M, El-Zein K, Gallego-Flores T, Perez AM, Jung SY, Botas J, Zoghbi HY, Jankowsky JL. Cross-species genetic screens to identify kinase targets for APP reduction in Alzheimer's disease. Hum Mol Genet. 2019 06 15; 28(12):2014-2029. PMID: 30753434; PMCID: PMC6548227.
      Citations: 1     Fields:    Translation:HumansAnimalsCells
    21. Rousseaux MWC, Al-Ramahi I, Jeong HH, Bajic A, Revelli JP, Ye H, Phan ET, Deger JM, Perez AM, Kim JY, Lavery LA, Xu Q, Li MZ, Kang H, Kim JJ, Shulman JM, Westbrook TF, Elledge SJ, Liu Z, Botas J, Zoghbi HY, V?zquez-V?lez GE. A Druggable Genome Screen Identifies Modifiers of a-Synuclein Levels via a Tiered Cross-Species Validation Approach. J Neurosci. 2018 10 24; 38(43):9286-9301. PMID: 30249792; PMCID: PMC6199406.
      Citations: 15     Fields:    Translation:HumansAnimalsCells
    22. Al-Ramahi I, Lu B, Di Paola S, Pang K, de Haro M, Peluso I, Gallego-Flores T, Malik NT, Erikson K, Bleiberg BA, Avalos M, Fan G, Rivers LE, Laitman AM, Hild M, Palacino J, Liu Z, Medina DL, Botas J, Diaz-Garc?a JR. High-Throughput Functional Analysis Distinguishes Pathogenic, Nonpathogenic, and Compensatory Transcriptional Changes in Neurodegeneration. Cell Syst. 2018 07 25; 7(1):28-40.e4. PMID: 29936182; PMCID: PMC6082401.
      Citations: 13     Fields:    Translation:HumansAnimalsCells
    23. Al-Ramahi I, Giridharan SSP, Chen YC, Patnaik S, Safren N, Hasegawa J, de Haro M, Wagner Gee AK, Titus SA, Jeong H, Clarke J, Krainc D, Zheng W, Irvine RF, Barmada S, Ferrer M, Southall N, Weisman LS, Botas J, Marugan JJ. Inhibition of PIP4K? ameliorates the pathological effects of mutant huntingtin protein. Elife. 2017 12 26; 6. PMID: 29256861; PMCID: PMC5743427.
      Citations: 21     Fields:    Translation:HumansAnimalsCells
    24. Rousseaux MW, de Haro M, Lasagna-Reeves CA, De Maio A, Park J, Jafar-Nejad P, Al-Ramahi I, Sharma A, See L, Lu N, Vilanova-Velez L, Klisch TJ, Westbrook TF, Troncoso JC, Botas J, Zoghbi HY. TRIM28 regulates the nuclear accumulation and toxicity of both alpha-synuclein and tau. Elife. 2016 10 25; 5. PMID: 27779468; PMCID: PMC5104516.
      Citations: 34     Fields:    Translation:HumansAnimalsCells
    25. Lasagna-Reeves CA, de Haro M, Hao S, Park J, Rousseaux MW, Al-Ramahi I, Jafar-Nejad P, Vilanova-Velez L, See L, De Maio A, Nitschke L, Wu Z, Troncoso JC, Westbrook TF, Tang J, Botas J, Zoghbi HY. Reduction of Nuak1 Decreases Tau and Reverses Phenotypes in a Tauopathy Mouse Model. Neuron. 2016 Oct 19; 92(2):407-418. PMID: 27720485; PMCID: PMC5745060.
      Citations: 48     Fields:    Translation:HumansAnimalsCells
    26. Soriano S, Calap-Quintana P, Al-Ramahi I, Botas J, Llorens JV, Guti?rrez L, Mart?nez-Sebasti?n MJ, Molt? MD. Metal Homeostasis Regulators Suppress FRDA Phenotypes in a Drosophila Model of the Disease. PLoS One. 2016; 11(7):e0159209. PMID: 27433942; PMCID: PMC4951068.
      Citations: 14     Fields:    Translation:HumansAnimalsCells
    27. Langfelder P, Cantle JP, Chatzopoulou D, Wang N, Gao F, Al-Ramahi I, Lu XH, Ramos EM, El-Zein K, Zhao Y, Deverasetty S, Tebbe A, Schaab C, Lavery DJ, Howland D, Kwak S, Botas J, Aaronson JS, Rosinski J, Coppola G, Horvath S, Yang XW. Integrated genomics and proteomics define huntingtin CAG length-dependent networks in mice. Nat Neurosci. 2016 Apr; 19(4):623-33. PMID: 26900923; PMCID: PMC5984042.
      Citations: 148     Fields:    Translation:Animals
    28. Hangen E, Poizat G, Al-Ramahi I, Pardo R, Bourg N, Souquere S, Botas J, Humbert S, El-Daher MT, Bruy?re J, Mayet C, Pierron G, L?v?que-Fort S, Saudou F. Huntingtin proteolysis releases non-polyQ fragments that cause toxicity through dynamin 1 dysregulation. EMBO J. 2015 Sep 02; 34(17):2255-71. PMID: 26165689; PMCID: PMC4585462.
      Citations: 41     Fields:    Translation:HumansAnimalsCells
    29. Calap-Quintana P, Soriano S, Al-Ramahi I, Botas J, Llorens JV, Molt? MD, Mart?nez-Sebasti?n MJ. TORC1 Inhibition by Rapamycin Promotes Antioxidant Defences in a Drosophila Model of Friedreich's Ataxia. PLoS One. 2015; 10(7):e0132376. PMID: 26158631; PMCID: PMC4497667.
      Citations: 22     Fields:    Translation:HumansAnimals
    30. Yao Y, Cui X, Al-Ramahi I, Sun X, Li B, Hou J, Difiglia M, Palacino J, Wu ZY, Ma L, Botas J, Lu B. A striatal-enriched intronic GPCR modulates huntingtin levels and toxicity. Elife. 2015 Mar 04; 4. PMID: 25738228; PMCID: PMC4372774.
      Citations: 34     Fields:    Translation:HumansAnimalsCells
    31. Lu XH, Mattis VB, Wang N, Al-Ramahi I, van den Berg N, Fratantoni SA, Waldvogel H, Greiner E, Osmand A, Elzein K, Xiao J, Dijkstra S, de Pril R, Vinters HV, Faull R, Signer E, Kwak S, Marugan JJ, Botas J, Fischer DF, Svendsen CN, Munoz-Sanjuan I, Yang XW. Targeting ATM ameliorates mutant Huntingtin toxicity in cell and animal models of Huntington's disease. Sci Transl Med. 2014 Dec 24; 6(268):268ra178. PMID: 25540325.
      Citations: 57     Fields:    Translation:HumansAnimalsCells
    32. Park J, Al-Ramahi I, Tan Q, Mollema N, Diaz-Garcia JR, Gallego-Flores T, Lu HC, Lagalwar S, Duvick L, Kang H, Lee Y, Jafar-Nejad P, Sayegh LS, Richman R, Liu X, Gao Y, Shaw CA, Arthur JSC, Orr HT, Westbrook TF, Botas J, Zoghbi HY. RAS-MAPK-MSK1 pathway modulates ataxin 1 protein levels and toxicity in SCA1. Nature. 2013 Jun 20; 498(7454):325-331. PMID: 23719381; PMCID: PMC4020154.
      Citations: 74     Fields:    Translation:HumansAnimalsCells
    33. de Haro M, Al-Ramahi I, Jones KR, Holth JK, Timchenko LT, Botas J. Smaug/SAMD4A restores translational activity of CUGBP1 and suppresses CUG-induced myopathy. PLoS Genet. 2013 Apr; 9(4):e1003445. PMID: 23637619; PMCID: PMC3630084.
      Citations: 15     Fields:    Translation:HumansCells
    34. Lu B, Al-Ramahi I, Valencia A, Wang Q, Berenshteyn F, Yang H, Gallego-Flores T, Ichcho S, Lacoste A, Hild M, Difiglia M, Botas J, Palacino J. Identification of NUB1 as a suppressor of mutant Huntington toxicity via enhanced protein clearance. Nat Neurosci. 2013 May; 16(5):562-70. PMID: 23525043.
      Citations: 47     Fields:    Translation:HumansAnimalsCells
    35. Miller JP, Yates BE, Al-Ramahi I, Berman AE, Sanhueza M, Kim E, de Haro M, DeGiacomo F, Torcassi C, Holcomb J, Gafni J, Mooney SD, Botas J, Ellerby LM, Hughes RE. A genome-scale RNA-interference screen identifies RRAS signaling as a pathologic feature of Huntington's disease. PLoS Genet. 2012; 8(11):e1003042. PMID: 23209424; PMCID: PMC3510027.
      Citations: 23     Fields:    Translation:HumansAnimalsCells
    36. Shirasaki DI, Greiner ER, Al-Ramahi I, Gray M, Boontheung P, Geschwind DH, Botas J, Coppola G, Horvath S, Loo JA, Yang XW. Network organization of the huntingtin proteomic interactome in mammalian brain. Neuron. 2012 Jul 12; 75(1):41-57. PMID: 22794259; PMCID: PMC3432264.
      Citations: 135     Fields:    Translation:HumansAnimals
    37. Zhang N, Li B, Al-Ramahi I, Cong X, Held JM, Kim E, Botas J, Gibson BW, Ellerby LM. Inhibition of lipid signaling enzyme diacylglycerol kinase epsilon attenuates mutant huntingtin toxicity. J Biol Chem. 2012 Jun 15; 287(25):21204-13. PMID: 22511757; PMCID: PMC3375542.
      Citations: 11     Fields:    Translation:HumansAnimalsCells
    38. Miller JP, Holcomb J, Al-Ramahi I, de Haro M, Gafni J, Zhang N, Kim E, Sanhueza M, Torcassi C, Kwak S, Botas J, Hughes RE, Ellerby LM. Matrix metalloproteinases are modifiers of huntingtin proteolysis and toxicity in Huntington's disease. Neuron. 2010 Jul 29; 67(2):199-212. PMID: 20670829; PMCID: PMC3098887.
      Citations: 86     Fields:    Translation:HumansAnimalsCells
    39. Al-Ramahi I, Benito-Sipos J, Veenstra JA, Herrero P, de Haro M, L?pez-Arias B, Dorado B. Detailed analysis of leucokinin-expressing neurons and their candidate functions in the Drosophila nervous system. Cell Tissue Res. 2010 Feb; 339(2):321-36. PMID: 19941006.
      Citations: 36     Fields:    Translation:AnimalsCells
    40. Al-Ramahi I, Lim J, Zhang M, Sorensen R, de Haro M, Branco J, Pulst SM, Zoghbi HY, Botas J, P?rez AM. dAtaxin-2 mediates expanded Ataxin-1-induced neurodegeneration in a Drosophila model of SCA1. PLoS Genet. 2007 Dec 28; 3(12):e234. PMID: 18166084; PMCID: PMC2323314.
      Citations: 53     Fields:    Translation:Animals
    41. Branco J, Al-Ramahi I, Ukani L, Fernandez-Funez P, Botas J, P?rez AM, Rinc?n-Limas D. Comparative analysis of genetic modifiers in Drosophila points to common and distinct mechanisms of pathogenesis among polyglutamine diseases. Hum Mol Genet. 2008 Feb 01; 17(3):376-90. PMID: 17984172.
      Citations: 43     Fields:    Translation:HumansAnimals
    42. Al-Ramahi I, Lam YC, Chen HK, de Gouyon B, Zhang M, Branco J, de Haro M, Patterson C, Zoghbi HY, Botas J, P?rez AM. CHIP protects from the neurotoxicity of expanded and wild-type ataxin-1 and promotes their ubiquitination and degradation. J Biol Chem. 2006 Sep 08; 281(36):26714-24. PMID: 16831871.
      Citations: 90     Fields:    Translation:HumansAnimalsCells
    43. Al-Ramahi I, De Gouyon B, Ukani L, Rosa A, Ashizawa T, Cooper TA, Botas J, de Haro M, Faustino NA. MBNL1 and CUGBP1 modify expanded CUG-induced toxicity in a Drosophila model of myotonic dystrophy type 1. Hum Mol Genet. 2006 Jul 01; 15(13):2138-45. PMID: 16723374.
      Citations: 69     Fields:    Translation:HumansAnimalsCells
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